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FRI0243 Extent of disease on high-resolution CT lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease
  1. O.A. Moore1,
  2. N. Goh2,
  3. T. Corte3,
  4. H. Rouse4,
  5. O. Hennessy4,
  6. V. Thakkar1,
  7. J. Byron1,
  8. J. Sahhar5,
  9. J. Roddy6,
  10. P. Youssef7,
  11. P. Nash8,
  12. J. Zochling9,
  13. S.M. Proudman10,
  14. W. Stevens1,
  15. M. Nikpour1
  16. and Australian Scleroderma Interest Group
  1. 1Rheumatology, St Vincent’s Hospital
  2. 2Respiratory Medicine, Austin Hospital, Melbourne
  3. 3Respiratory Medicine, Royal Prince Alfred Hospital, Sydney
  4. 4Radiology, St Vincent’s Hospital
  5. 5Rheumatology, Monash Medical Centre, Melbourne
  6. 6Rheumatology, Royal Perth Hospital, Perth
  7. 7Rheumatology, Royal Prince Alfred, Sydney
  8. 8Research Unit, Sunshine Coast Rheumatology, Maroochydore
  9. 9Rheumatology, The Menzies Institute, Hobart
  10. 10Rheumatology, Royal Adelaide Hospital, Adelaide, Australia


Objectives We sought to determine whether the extent of disease on high-resolution CT lung (HRCT), measured using a simple grading system,[1] is predictive of decline and mortality in systemic sclerosis-related interstitial lung disease (SSc-ILD), independently of pulmonary function tests (PFTs) and other prognostic variables.

Methods SSc patients with a baseline HRCT performed at the time of ILD diagnosis were identified. All PFTs performed during follow-up were retrieved. Demographic and disease-related data were prospectively collected. HRCTs were graded according to the percentage of lung disease seen; >20% - extensive, <20% - limited, unclear - indeterminate. Indeterminate HRCTs were converted to limited or extensive using an FVC threshold of 70%. The composite outcome variable was deterioration (defined as need for home oxygen or lung transplantation), or death.

Results Among 172 patients followed for mean±SD of 3.47±2.93 years, there were 33 outcome events. Figure 1 shows a Kaplan-Meier survival curve of HRCT score against survival. Survival estimates were significant for two raters and approached significance for the third (log-rank p<0.0001, p=0.0005, p=0.13). In Weibull multivariable hazards regression modelling, baseline HRCT grade was independently predictive of outcome, with an adjusted hazard ratio (aHR) =3.0, 95% CI: 1.2-7.5, p=0.02.

Conclusions Extensive changes (>20%) on HRCT at baseline, reported using a semi-quantitative grading system, are associated with a three-fold increased risk of deterioration or death in SSc-ILD, compared with limited changes.

  1. Goh NSL, Desai SR, Veeraraghavan S, et al. Interstitial Lung Disease in Systemic Sclerosis: A Simple Staging System. American Journal of Respiratory and Critical Care Medicine 2008;177:1248–1254.

Disclosure of Interest None Declared

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